A Study of Congenital Cranial Encephalocele Cases in Egypt

Abstract

Background: Congenital cranial encephalocele, a neural tube defect characterized by the herniation of intracranial contents through a cranial vault defect, represents a significant public health challenge in developing nations. In these settings, its epidemiology is distinct, and patient outcomes are disproportionately poor. This review, augmented by original findings from a clinical cohort in Egypt, investigates the interplay between regional risk factors notably consanguinity, folic acid deficiency, and limited prenatal care and the resultant disease burden. It further evaluates the role of surgical intervention as a vital determinant of neurodevelopmental prognosis. Our analysis identified critical determinants of surgical outcome, including specific preoperative, intraoperative, and postoperative factors. The development of postoperative hydrocephalus was a pivotal complication. Notably, poor long-term outcomes delayed development or mortality in cases of our cohort were strongly predicted by the presence of concomitant cranial anomalies (e.g., Chiari malformation, corpus callosum agenesis, and Dandy-Walker malformation), large sac size, and significant intraoperative challenges. The evidence underscores the necessity of a dual strategy: aggressive public health initiatives for primary prevention must be coupled with the strategic strengthening of specialized neurosurgical and neurocritical care services. This integrated approach is essential to mitigate the substantial morbidity and mortality associated with this condition in resource-limited settings.